Spontaneous bladder rupture in non-augmented bladder exstrophy

SummaryObjectiveBladder perforation is not commonly described in bladder exstrophy patients without bladder augmentation. The goal of this study was to identify the risk factors of spontaneous perforation in non-augmented exstrophy bladders.MethodsThe study was a retrospective multi-institutional review of bladder perforation in seven male and two female patients with classic bladder exstrophy-epispadias (E-E).ResultsCorrection of E-E was performed using Kelly repair in two and staged repair in seven (Table). Bladder neck repair was performed in eight patients at a mean age of 6 years. Three patients had additional urethral surgery. Before rupture, six patients were voiding only per urethra. Two patients were voiding urethrally but were also performing occasional CIC via a Mitrofanoff. One patient was performing CIC 3 hourly per urethra. Six were dry during the day. Six of the patients had lower urinary tract symptoms: five had frequency and four were straining to void. Two had suffered episodes of urinary retention. Pre-rupture ultrasound showed that the upper urinary tract was dilated in four patients. Micturating cystourethrogram was performed in six showing vesico-ureteral reflux in five. Two had urethral stenosis. Nuclear medicine was done in three patients with two abnormal differential function. Urodynamics was performed in two patients with low capacity (100 mL) and hypocompliant (<10) bladders. Both had high leak point pressures: 60 cmH2O at 100 mL. The mean age at rupture was 11 years, with a range of 5-20 years. Patients presented with abdominal pain, associated with signs of intestinal obstruction in seven and fever in two. Eight patients underwent laparotomy and one prolonged drainage via SPC. Simple closure was performed in seven and bladder neck closure in one, because of extension of the rupture inferiorly. All patients recovered well. Following rupture, five underwent augmentation and Mitrofanoff. One of these suffered a recurrent rupture. Two other patients refused augmentation and Mitrofanoff and one of these has since had a subsequent rupture.ConclusionsThe limitations of this series include the small number of patients and its retrospective nature, without knowledge of the incidence. Bladder rupture is a risk even in non-augmented bladder exstrophy. It is potentially life-threatening and most often requires laparotomy. Rupture occurs because of poor bladder emptying and/or high pressure. Urodynamics may identify those at risk. CIC with or without augmentation should not be delayed once poor bladder emptying and/or high pressure are identified.Table. Patients with bladder perforation.PtPre-rupture imagingPre-rupture continenceAge, yearsManagementOutcome1Small right kidney with high-grade VUR and high PVR90 min voiding with episodes of retention13Laparotomy IDC 3 weeksRefused augmentation and Mitrofanoff. Recurrent bladder rupture2Small left kidney (15% df) with high-grade VUR and high PVR. Poorly compliant bladder on urodynamicsStraining to void with frequency8Laparotomy and bladder neck closure with SPCAugmentation and Mitrofanoff3Trabeculated bladderStraining to void. High PVR. Incontinent5Laparotomy SPC 3 weeksRefused Mitrofanoff. Ongoing straining and lost to f/u4Trabeculated bladder, low-grade VUR bilaterally and urethral stenosis2-3 hourly voiding with straining. High PVR20SPC with prolonged drainageNo further surgery or recurrences known5Bilateral HUN3 hourly voiding with high PVR15Laparotomy IDC 3 weeksNo further surgery or recurrences known6Normal USSVoiding and dry on desmopressin and oxybutynin11Laparotomy IDC 3 weeksAugmentation with Mitrofanoff7Bilateral HUN with high-grade VUR. Poorly compliant bladder on urodynamicsCIC 3 hourly9Laparotomy SPCAugmentation with Mitrofanoff. Bladder stone post augment8Bilateral HUN with high-grade VUR and reduced function on leftVoiding with day and night wetting. Occasional CIC via Mitrofanoff8Laparotomy. Drainage via MitrofanoffAugmentation. Recurrent rupture near the anastomosis of the augment9Normal USS. Bladder capacity 100 mL. Low complianceVoiding with day and night leaking. Occasional CIC via Mitrofanoff10Laparotomy. Drainage via MitrofanoffAugmentation, redo Mitrofanoff (Monti)CIC, clean intermittent catheterization; HUN, hydroureteronephrosis; IDC, indwelling catheter; Pt, patient; PVR, post void residual; R, rupture; SPC, suprapubic catheter; USS, ultrasound; VUR, vesicoureteric reflux.